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Meningocele, Cranial - Frontoethmoidal

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Cephalocele is the protrusion of intracranial contents through a bony defect in the skull. Cranial meningocele refers to herniation of meninges only. Originally considered as one of the varied manifestations of failed neural tube closure such as anencephaly or spina bifida, epidemiologic data suggests that isolated frontoethmoidal cephalocele is a pathogenetically distinct condition. Families with this malformation are not at increased risk for neural tube defect in subsequent offspring, but may present with other central nervous system anomalies (6%). Thailand has an unusually high incidence of frontal cephaloceles.A sac protruding through an anterior cranial defect may be seen on a sagittal or transverse views through the orbital region. Hypertelorism may result. Although most fronto-ethmoidal cephaloceles are sporadic, occasionally they occur in conjunction with known genetic syndromes, and may present with characteristic associated defects. For example, in Roberts syndrome, an autosomal recessive disorder, there may be associated growth restriction, limb reduction defects, cleft lip and/or palate. Title: Cephalocele detection in utero: sonographic and clinical features Author: Budorick NE, Pretorius DH, McGahan JP, Grafe MR, James HE, Slivka J Journal: Ultrasound Obstet Gynecol 5: 77-85 Title: Prenatal diagnosis of an epidermal scalp cyst simulating an encephalocoele. Author: Shahabi S; Busine A Journal: Prenat Diagn Apr;18(4):373-7 Year: 1998 Title: Prenatally diagnosed neural tube defects: ultrasound, chromosome, and autopsy or postnatal findings in 212 cases. Author: Kennedy D; Chitayat D; Winsor EJ; Silver M; Toi A Journal: Am J Med Genet May 26;77(4):317-21 Year: 1998 Title: Neural tube defects in chromosomally normal and abnormal human embryos. Author: Coerdt W; Miller K; Holzgreve W; Rauskolb R; Schwinger E; Rehder H Journal: Ultrasound Obstet Gynecol Dec;10(6):410-5 Year: 1997 Title: Prenatal diagnosis and pediatric neurosurgery. Author: Bell WO; Nelson LH; Block SM; Rhoney JC Journal: Pediatr Neurosurg;24(3):134-7; discussion 138 Year: 1996

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